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. 2014 Sep 1;32(25):2788-93.
doi: 10.1200/JCO.2013.53.1996. Epub 2014 Jul 14.

Developmental and adaptive functioning in children with retinoblastoma: a longitudinal investigation

Victoria W Willard  1 , Ibrahim Qaddoumi  1 , Si Chen  1 , Hui Zhang  1 , Rachel Brennan  1 , Carlos Rodriguez-Galindo  1 , Matthew W Wilson  1 , Sean Phipps  2
Affiliations

Developmental and adaptive functioning in children with retinoblastoma: a longitudinal investigation

Victoria W Willard et al. J Clin Oncol. .

Abstract

Purpose: To determine the developmental trajectory of early cognitive and adaptive skills in young children with retinoblastoma from diagnosis to 5 years of age.

Patients and methods: Ninety-four patients with retinoblastoma treated according to an institutional protocol underwent serial assessments of cognitive and adaptive functioning at age 6 months and 1, 2, 3, and 5 years. Data were analyzed by treatment strata, with patients with 13q deletion analyzed separately.

Results: At baseline, across all patients (except those with 13q deletion), developmental functioning was comparable with the normative mean, with mean scores for all strata within the average range. However, at age 5 years, developmental functioning was in the low average range and significantly below normative means. The trajectories of developmental functioning demonstrated significant decline over time, although this varied by treatment group/strata. Patients treated with enucleation only evidenced the greatest decline in cognitive functioning; significant change was not observed in patients treated with other modalities. Notable declines in parent-reported communication skills were observed in the majority of patients. Patients with 13q deletion evidenced delayed cognitive functioning at baseline, but minimal declines were observed through age 3 years. However, significant decreases in adaptive functioning were demonstrated over time for the 13q deletion subset.

Conclusion: The declines in functioning observed in this study were unexpected, as was the poorer performance of the enucleation-only group. This highlights the necessity of continuing to assess cognitive functioning in patients with retinoblastoma as they age. Additional research is necessary to determine the long-term trajectory of cognitive development in this population.

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Conflict of interest statement

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

Figures

Fig 1.
Fig 1.
Longitudinal trajectory of the Early Learning Composite, delineated by strata.
Fig 2.
Fig 2.
Longitudinal trajectory of the Adaptive Behavior Composite, delineated by strata.
See this image and copyright information in PMC

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